Congenital heart diseaseCohort study

Pulmonary Artery Hypoplasia in Neonates With Tetralogy of Fallot

J Am Coll CardiolPublished

  • Retrospective cohort study
  • multicentre registry (Congenital Cardiac Research Collaborative)

Authors' conclusion

Despite perception, the presence of hPA in neonates with sTOF conferred no increase in overall hazard of mortality or reintervention after definitive repair. PA growth was superior in the hPA cohort.

Study context

Population
Neonates with symptomatic tetralogy of Fallot, 2005–2017
Participants
542 neonates
Exposure
Hypoplastic pulmonary artery (PAs) vs normal PAs
  • Hypoplastic pulmonary arteries (hPA)n = 188
  • Normal PAn = 354reference
Outcome
Mortality
Follow-up
Median 4.1 years

Main finding

As reported in the abstract

Survival

Survival similar between groups (unadjusted)

P = 0.16

Survival similar between groups (adjusted)

P = 0.25

Observational data: associations, not effects.

Secondary outcomes

OutcomeHR (95% CI)P value
Reintervention · overall1.28 (1.01–1.63)P = 0.044
Reintervention · after definitive repair1.21 (0.93–1.58)P = 0.16

Staged repair (vs primary TOF repair)

  • Staged repair (vs primary TOF repair): P = 0.07

PA growth · 1 year

Greater PA growth in hPA cohort, particularly right PA
P < 0.001

Original publication

Pulmonary Artery Hypoplasia in Neonates With Tetralogy of Fallot. J Am Coll Cardiol. Published 15 August 2023. doi:10.1016/j.jacc.2023.05.051

Summarised from the published abstract; every number is checked against it